A Devon family fighting a ‘financial battle’ to make a potentially life-saving drug available for people with cystic fibrosis (CF) have started an online campaign which has been the fastest growing UK government petition at times.

In just two months, more than 40,000 have signed the petition which is calling for the NHS to supply Orkambi, which is already available in 11 other EU countries. Case studies report miraculous improvements in health, and it is believed it could greatly improve the life expectancy of people with CF.

The petition was launched by the Meredith family who know all too well the difference having the drug could make. CF is a genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine.

Jessica’s younger sister Sarah, 26, who lives in Totnes, and has what is known as ‘complicated CF’ because it is combined with alpha 1 antitrypsin deficiency disease.

Jessica Meredith, left, and her sister Sarah

Together they have caused her to have insulin dependent diabetes, advanced cirrhosis with portal hypertension, pancreatic insufficiency, gastrointestinal problems with chronic nausea, low bone density and joint problems, as well as the usual lung deterioration that is the hallmark of CF.

For Jessica, 32, of Exeter, it has meant that she has to study and work near to Sarah, and has reduced her working hours to allow time to help Sarah manage her huge treatment burden and travel to hospital appointments in Birmingham, Liverpool and London, as well as local centres in Devon.

Currently Sarah is on medications which help to manage the decline in her health as the disease progresses.

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Jessica, who took part in an Orkambi protest march in London last year, said: “Children and young adults with CF endure lifelong suffering and early death. They need Orkambi and other precision medicines as they are developed.

“Sufferers in the EU, US and Australia can access the drugs, but not the UK. Hundreds have died in the three years since these drugs were licensed.

“All MPs who debated the petition in March were passionately in favour of supplying the drugs. Despite an ever-increasing abundance of evidence as to the drugs’ effectiveness, CF sufferers still do not have access.”

She added: “Consultants nationwide have asked the NHS to make the drugs available. Doctors have expressed distress at seeing children die whilst the drug they need is on the shelf. After three years, conclude negotiations and fund these drugs.

“The health select committee including MPs Sarah Wollaston, Ben Bradshaw and Johnny Mercer, have raised an inquiry into this issue.”

Exeter MP Ben Bradshaw said: “I continue to hope that the drugs’ manufacturer, Vertex, will accept the price offered by the NHS and stop this wrangling, so that my constituents in Exeter and around the country can get this medicine they so desperately need.

"My colleagues and I will be questioning those involved in this decision when they appear before the Health Select Committee next month.”

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Orkambi is manufactured by Vertex Pharmaceuticals. NHS England and Vertex have been at loggerheads over reaching a deal to make the drug available.

Vertex has confirmed it will be participating in the forthcoming public hearing of the Health and Social Care Committee (HSC) of the House of Commons on March7.

A spokesperson for Vertex said: “The hearing will discuss the availability of our cystic fibrosis medicines. Out of respect for the parliamentary process, we will not be providing further updates at this time.

“Vertex welcomes the committee’s interest in access to cystic fibrosis medicines and we look forward to continuing to support its inquiry into this important issue.”

A spokesperson for NHS England said: “We understand how difficult it must be for families affected by cystic fibrosis. This is why it is so important that as with all companies seeking NHS approval for treatments of this kind, Vertex must fully engage with the NICE process.”